Diagnostic Revision From Primary Lateral Sclerosis to Amyotrophic Lateral Sclerosis: A Cohort Study - PubMed
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- #primary lateral sclerosis
- #amyotrophic lateral sclerosis
- #diagnostic revision
- Primary lateral sclerosis (PLS) is an upper motor neuron syndrome diagnosed after excluding other conditions like ALS, with criteria requiring 2 years (probable PLS) or 4 years (definite PLS) of disease duration.
- A cohort study of 478 PLS patients found 18% (88 cases) were later re-diagnosed with ALS, with 86% of revisions occurring before 4 years.
- Factors linked to diagnostic revision from PLS to ALS included older age at onset (63.4 vs. 58.0 years), bulbar onset (38.6% vs. 19.7%), and faster disease progression (ALS Functional Rating Scale slope 0.43 vs. 0.18).
- Higher risk of revision was associated with faster progression (subdistribution hazard ratio 3.08) and having probable vs. definite PLS (SHR 3.08).
- The study suggests incorporating progression rate into diagnostic criteria could improve accuracy and identify patients at greater risk of developing ALS.